Bruna Ziegler

Prevalência de hipertensão pulmonar avaliada por ecocardiografia Doppler em uma população de pacientes adolescentes e adultos com fibrose cística

Objective: To determine the prevalence of pulmonary hypertension (PH) in patients with cystic fibrosis (CF), to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. Methods: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older) enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. Results: Tricuspid regurgitant jet velocity (TRV) was obtained in 37 of the 40 patients studied. The prevalence of PH was 49% with a TRV cut-off of 2.5 m/s (18 patients) and 30% with a TRV cut-off of 2.8 m/s (11 patients). Peripheral oxygen saturation (SpO 2 ) at rest, clinical score, forced expiratory volume in one second (FEV 1 ), and forced vital capacity (FVC) were significantly lower in the group with PH. The TRV was found to correlate significantly with SpO 2 at rest (p < 0.001), clinical score (p < 0.001), radiographic score (p = 0.030), FEV 1 in liters (p < 0.001) and in % of predicted (p < 0.001), and FCV in liters (p = 0.008) and in % of predicted (p = 0.001). The single best predictor of TRV was SpO 2 at rest (p < 0.001). Conclusion: The high prevalence of PH in the CF patients studied suggests that PH should be considered in the evaluation and follow-up treatment of such patients. The best predictor of PH was SpO 2 at rest.

Exercise programme in patients with cystic fibrosis: A randomized controlled trial

OBJECTIVES Assess the effects of a home exercise programme, based on aerobic training and muscle strength training, in patients with cystic fibrosis (CF), for a period of 3 months. METHODS Randomised controlled clinical experiment, with an analysis of intention to treat including clinically stable patients with CF and of age ≥ 16. Assessments include: a 6 min walk test (6 MWT), one-repetition maximum strength test (1 RM), spirometry and quality of life questionnaires. The patients randomised for the exercise group exercise group followed a home exercise protocol, supervised by telephone, while the control group maintained their usual activities. RESULTS 41 Patients were included, 22 in the control group and 19 in the exercise group. The exercise group presented a significant increase in muscle strength in upper limbs (UULL) on the 1 RM test. There was no significant difference between groups on the scores for general quality of life and specifically for CF and in the distance walked on the 6 MWT. CONCLUSION The study demonstrated that a home exercise programme had positive effects in adult patients with CF, including gain in muscle strength in UULL. No increase in tolerance to exercise was shown and improvement in the quality of life of the patients who received intervention.

Adherence to Airway Clearance Therapies by Adult Cystic Fibrosis Patients

BACKGROUND: Airway clearance therapy (ACT) is critical in cystic fibrosis (CF). OBJECTIVES: To determine rates of self-reported adherence to ACT by patients treated in an adult CF program, to identify patient characteristics associated with poor adherence, to typify adherence according to ACT technique, and to indicate reasons for poor adherence. METHODS: Our cross-sectional study included CF subjects age 16 years and older. Enrollees were evaluated via general structured questionnaire, adherence questionnaire, clinical assessment, spirometry, and SpO2 values. Each was stratified by self-reporting protocol as high, moderate, or poor adherence to ACT. Concordance between physiotherapist recommended ACT technique and self-reported subject adherence was subjected to agreement analysis. RESULTS: Of the 63 subjects studied, 38 (60%) qualified as high adherence, 12 (19%) as moderate adherence, and 13 (21%) as poor adherence. Logistic regression identified education level (less than high school) as an independent factor associated with poor adherence (odds ratio 10.2, 95% CI 1.23–84.7, P = .03). Positive expiratory pressure (κ = 0.87) and flutter device (κ = 0.63) usage both corresponded with a high level of agreement, while active cycle of breathing technique (κ = 0.40) and autogenic drainage (κ = 0.39) each showed moderate agreement. Agreement was low for percussion and postural drainage (κ = 0.23). Reasons given most frequently for poor adherence to ACT were “not enough time to do ACT” (28%), “cannot be bothered” (16%), and “do not enjoy ACT technique” (8%). Many (32%) provided no reason. CONCLUSIONS: Study outcomes showed a high rate of ACT adherence in adult CF subjects. Lower level of education was the most important factor in poor adherence to ACT. Self-reported adherence and treatment recommendations were in best agreement with positive expiratory pressure and flutter device techniques.

Preditores da dessaturação do oxigênio no teste da caminhada de seis minutos em pacientes com fibrose cística

OBJECTIVE To identify the predictive factors of oxygen desaturation during the six-minute walk test (6MWT) in patients with cystic fibrosis (CF). METHODS Prospective cross-sectional study involving clinically stable patients with CF aged > or = 10 years. The patients were submitted to nutritional evaluations, oral glucose tolerance tests, pulmonary function tests, chest X-rays and 6MWTs. RESULTS The study included 88 patients (43 females and 45 males; mean age, 19.9 + or - 7.2 years; mean FEV(1), 65.4 + or - 28.4%). We observed oxygen desaturation in 13 patients (OD+ group) and no oxygen desaturation in 75 (ODmicro group). In comparison with ODmicro group patients, OD+ group patients presented higher mean age (p = 0.004), worse clinical score (p < 0.001), worse radiological score (p < 0.001), higher incidence of glucose intolerance (p = 0.004), lower incidence of methicillin-sensitive Staphylococcus aureus infection (p < 0.001), higher incidence of methicillin-resistant S. aureus infection (p = 0.016), higher incidence of Pseudomonas aeruginosa infection (p = 0.008), lower mean resting SpO(2) (p < 0.001) and lower mean FEV(1) (p < 0.001). In the logistic regression analysis, oxygen desaturation during the 6MWT correlated with resting SpO(2) (OR = 0.305, p < 0.001) and FEV(1) (OR = 0.882, p = 0.025). The parameters maximizing the predictive value for oxygen desaturation were resting SpO(2) < 96% and FEV(1) < 40%. In this sample, 15% of the patients with CF aged > or = 10 years presented oxygen desaturation during the 6MWT. CONCLUSIONS Resting SpO(2) < 96% and FEV(1) < 40% can predict oxygen desaturation during the 6MWT.

Padrões ventilatórios na espirometria em pacientes adolescentes e adultos com fibrose cística

OBJECTIVE To evaluate spirometric patterns of respiratory disorders and their relationship with functional severity and maximal expiratory flows at low lung volumes in patients with cystic fibrosis (CF). METHODS A retrospective cross-sectional study including adolescents and adults with CF. All of the patients were submitted to spirometry. Patients were classified as having preserved respiratory function, obstructive lung disease (OLD), OLD with reduced FVC, presumptive restrictive lung disease (RLD) or mixed obstructive and restrictive lung disease (MORLD). Maximal expiratory flows at low lung volumes were assessed using FEF(25-75%), FEF(75%) and FEF(75%)/FVC. We included 65 normal subjects, also submitted to spirometry, as a control group. RESULTS The study group included 65 patients: 8 (12.3%) with preserved lung function; 18 (27.7%) with OLD; 24 (36.9%) with OLD and reduced FVC; 5 (7.7%) with presumptive RLD; and 10 (15.4%) with MORLD. The FEV1 was significantly lower in the OLD with reduced FVC group and the MORLD group than in the other groups (p < 0.001). In the patients with preserved respiratory function, FEF(25-75%) and FEF(75%) were significantly reduced in 1 patient, as was FEF(75%)/FVC in 2 patients. CONCLUSIONS The respiratory pattern was impaired in 88% of the patients with CF. The most common pattern was OLD with reduced FVC. The degree of functional impairment was greater in the OLD with reduced FVC group and in the MORLD group than in the other groups. Maximal expiratory flows at low lung volumes were impaired in a low percentage of patients with preserved respiratory function.

Glucose Intolerance in Patients With Cystic Fibrosis: Sex-Based Differences in Clinical Score, Pulmonary Function, Radiograph Score, and 6-Minute Walk Test

OBJECTIVE: To determine the sex-specific relationships between glucose intolerance and Shwachman-Kulczycki clinical score, lung function, Brasfield chest-radiograph score, and exercise capacity in patients with cystic fibrosis (CF). METHODS: We used a cross-sectional study design and included CF patients ≥ 10 years old. All patients had clinical and nutritional evaluation, oral glucose tolerance test, spirometry, chest radiograph, and 6-min walk test (6MWT). Patients were classified as having normal glucose tolerance, impaired glucose tolerance, or CF-related diabetes mellitus. RESULTS: We included 88 patients: 59 with normal glucose tolerance, 15 with impaired glucose tolerance, and 14 with CF-related diabetes. Shwachman-Kulczycki clinical score (P = .04), at-rest SpO2 (P = .001), SpO2 difference before versus after 6MWT (P = .001), and Brasfield chest-radiograph score (P = .01) were significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group, but did not differ from the CF-related-diabetes group. In female patients only, percent-of-predicted FEV1 was significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group (P = .02), but did not differ from the CF-related-diabetes group (P = .10). There was a significant relationship between glucose intolerance and sex when clinical score, 6-min walk distance, FEV1, and radiograph score were combined in a multivariate analysis. CONCLUSIONS: In patients with CF, glucose intolerance was associated with poor clinical score, lower at-rest SpO2, greater SpO2 difference before versus after 6MWT, poor lung function, and lower radiograph score. Overall, multivariate analysis indicated poorer performance in the latter variables in female patients with glucose intolerance than in male patients with glucose intolerance.

Variability of the perception of dyspnea in healthy subjects assessed through inspiratory resistive loading

Objective: Few studies have evaluated the variability of the perception of dyspnea in healthy subjects. The objective of this study was to evaluate the variability of the perception of dyspnea in healthy subjects during breathing against increasing inspiratory resistive loads, as well as to assess the association between the level of perception of dyspnea and the level of physical activity. Methods: This was a cross-sectional study involving healthy individuals 16 years of age or older. Subjects underwent inspiratory resistive loading testing, in which the level of perception of dyspnea was quantified with the modified Borg scale. We also determined body mass indices (BMIs), assessed maximal respiratory pressures, performed pulmonary function tests, applied the international physical activity questionnaire (IPAQ)-long form, and conducted six-minute walk tests (6MWTs). The level of perception of dyspnea was classified as low (Borg score < 2), intermediate (Borg score, 2-5), or high (Borg score > 5). Results: We included 48 healthy subjects in the study. Forty-two subjects completed the test up to a load of 46.7 cmH2O/L/s. The level of perception of dyspnea was classified as low, intermediate, and high in 13, 19, and 10 subjects, respectively. The level of perception of dyspnea was not significantly associated with age, gender, BMI, IPAQ-long form score, maximal respiratory pressures, or pulmonary function test results. Conclusions: The scores for perceived dyspnea induced by inspiratory resistive loading in healthy subjects presented wide variability. The perception of dyspnea was classified as low in 31% of the subjects, intermediate in 45%, and high in 24%. There was no association between the level of perception of dyspnea and the level of physical activity (IPAQ or six-minute walk distance).

Infecção bacteriana crônica e indicadores ecocardiográficos de hipertensão pulmonar em pacientes com fibrose cística

Objectives: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). Methods: A prospective cross-sectional study involving CF patients (≥16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 ± 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. Results: In terms of the following variables, no significant differences were found between P. aeruginosa-positive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV 1 ; p = 0.693), radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/ pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV 1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). Conclusions: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

Dyspnea perception in cystic fibrosis patients

We evaluated dyspnea perception in cystic fibrosis patients compared with normal subjects, during an inspiratory resistive loading test and 6-min walk test. We also evaluated the correlation between dyspnea scores induced by resistive loads and by the 6-min walk test. In this prospective, cross-sectional study, 31 patients with cystic fibrosis (≥15 years of age) and 31 age-, gender-, and ethnicity-matched healthy volunteers (20 females and 11 males per group) underwent inspiratory resistive loading, spirometry, and the 6-min walk test. As the magnitude of the inspiratory loads increased, dyspnea scores increased (P<0.001), but there was no difference between groups in dyspnea score (P=0.654). Twenty-six (84%) normal subjects completed all the resistive loads, compared with only 12 (39%) cystic fibrosis patients (P<0.001). Dyspnea scores were higher after the 6-min walk test than at rest (P<0.001), but did not differ between groups (P=0.080). Post-6-min walk test dyspnea scores correlated significantly with dyspnea scores induced by resistive loads. We conclude that dyspnea perception induced in cystic fibrosis patients by inspiratory resistive loading and by 6-min walk test did not differ from that induced in normal subjects. However, cystic fibrosis patients discontinued inspiratory resistive loading more frequently. There were significant correlations between dyspnea perception scores induced by inspiratory resistance loading and by the 6-min walk test. This study should alert clinicians to the fact that some cystic fibrosis patients fail to discriminate dyspnea perception and could be at risk for delay in seeking medical care.

Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings.

In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance, and sleep quality.